Among the many unusual
diseases that infectious disease consultants encounter in the evaluation of
patients with fever of unknown origin (FUO) is Still's disease, a rare
rheumatologic condition associated with recurrent episodes of fever, evanescent
rash, and often, arthritis. Patients also have leukocytosis and unusually
dramatic elevations in ferritin. I just had the opportunity to see my second
case of Still's disease in three years, in an 18-year-old with fever, a
brilliant confluent erythematous rash (which is somewhat atypical for this
disease), severe limiting arthralgias, and a ferritin level of about 26,000 mg/dL.
A clue to the diagnosis was her history of two similar episodes during the
previous year, both of which spontaneously resolved, and one of which required
hospitalization and was attributed to a viral syndrome.

Because these kind of patients
are often referred to a rheumatologist once the diagnosis is made, I wasn't sure
what her long-term outcome might be. Well, here is the answer.

Ten patients with
juvenile-onset Still's disease (occurring before the age of 15), and eight
patients with adult Still's were followed for at least 10 years after their
initial presentation.1 Both groups had similar initial manifestations
and joint involvement, and equally severe long-term sequellae. All 18 patients
experienced multiple, recurrent flare-ups of disease requiring large doses of
corticosteroids. Patients with juvenile-onset Still's generally had few or no
sequellae until after age 15. Five of 10 (50%) of the pediatric patients
subsequently developed progressive narrowing of joint spaces and articular
destruction, and four required bilateral total hip replacements. Three pediatric
patients developed ankylosis of the spine, including one who developed severe
scoliosis. The articular changes in these patients generally occurred within 5-7
years of presentation, while most were still young adults. Two patients had
severe growth retardation as a result of their bone and joint involvement.

A similar frequency (50%) of
joint destruction occurred in adult-onset Still's subjects. Joint disease
occurred within 1-8 years of presentation in four of eight adult subjects, and
progressed rapidly despite the administration of aggressive anti-inflammatory
agents and corticosteroids. Within 10 years, three patients required five total
hip replacements, and another had severe subchondral erosion of the hip and
shoulder joints.

Comment by Carol A. Kemper, MD

While most of the systemic
manifestations of disease responded fairly well to therapy, these data show that
the joint destruction was very difficult to control in about one-half of the
patients. In particular, the initial cardiopulmonary manifestations (e.g.,
pericarditis) of disease did not recur. However, three of the patients with
adult Still's developed amyloidosis, resulting in renal failure in two and
gastrointestinal bleeding in one. Two of these three patients died as a
consequence of their amyloidosis. Iatrogenic complications were also frequent,
including aseptic arthritis and gastrointestinal bleeding from high-dose
anti-inflammatory agents. Because the use of high-dose anti-inflammatory agents
and corticosteroids did not halt the progression of joint destruction, which
progressed fairly rapidly once it appeared, Cabane and colleagues propose the
additional use of other agents, such as methotrexate.

Infectious disease consultants
may only see a handful of Still's disease cases in their careers, usually in the
evaluation of FUO, and we are seldom involved in the long-term care of these
patients once the diagnosis is made. Many of these patients do extremely poorly,
and half are at risk for severe joint destruction within 10 years of
presentation, irrespective of the age of onset of disease. In addition, these
data suggest that amyloidosis may occur in up to one-third of patients with
adult-onset Still's, often with a fatal outcome.

Reference

1. Cabane J, et al. Comparison
of long-term evolution of adult onset and juvenile onset Still's disease, both
followed up for more than 10 years. Ann Rheum Dis 1990;49:283-285.

Infectious disease
consultants usually encounter Still's disease in the evaluation of patients
with:

a. leukocytosis.

b. arthritis.

c. fever of unknown origin (FUO).

d. dramatic elevations in
ferritin.

The most common condition
that occurred in patients with juvenile-onset Still's disease and those with
adult-onset Still's disease is:

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This page was last updated on January 17, 2001